MRKH: When the uterus and vagina do not develop

MRKH stands for Mayer–Rokitansky–Küster–Hauser syndrome. Other names for this condition are müllerian aplasia and müllerian agenesis. MRKH occurs in one out of every 4,000–10,000 females.

MRKH is caused when the channels that normally form the fallopian tubes, uterus, cervix, and the upper two-thirds of the vagina do not get formed for various reasons. Other symptoms may include hearing loss and kidney and/or spinal problems (see illustration). There are variations in which of these organs are affected, but the ovaries, formed separately, are usually normal. Patients with MRKH have normal breast development, normal secondary sexual body proportions, body hair, and hymenal tissue.

MRKH may not become evident until a woman reaches puberty and menstruations do not occur (primary amenorrhea). If the first menstrual cycle has not occurred within three years of the onset of breast development, it is important to consult a gynecologist for further evaluation.

Before any treatment is recommended, however, it is important to consult a highly specialized gynecologist, one who is thoroughly trained in the management of müllerian anomalies and the potential problems that they may be associated with, to undergo as thorough an evaluation as possible. Academia’s Dr. Assia Stepanian is one such specialist.

Women who are born with MRKH can, and should, be treated. Their two major concerns are usually sexual intimacy and the inability to have children. In most cases, proper treatment can alleviate both of these concerns.

MRKH does not rule out the ability of a woman to have children since her ovaries are not affected. While she will never become pregnant or carry her own child, assisted reproductive techniques, including surrogate motherhood, may be used to enable a woman with MRKH to have her own, normal baby. There are many wonderful organizations, like Atlanta Fertility Support Alliance that are available to help women deal with the physical and emotional problems that infertility can cause.

What’s more, sexual intimacy can be greatly improved, even for women well beyond their teenage years, through the construction of a neovagina. In 90% of cases, women are able to achieve anatomic and functional success through a non-surgical technique called vaginal dilation.

There are, however, benefits to the surgical technique practiced by Academia’s Dr. Assia Stepanian. While vaginal dilation can be a long, sometimes painful, process, this minimally invasive surgical technique is the only type of surgery for vaginal agenesis that forms the neovagina of average size and width immediately without the need of grafts, tractional devices, or the formation of scars. Postoperative narcotics are used only minimally, no therapeutic dilation is required, and blood loss is usually minimal.

Dr. Stepanian is a daughter of L.V. Adamyan, the innovator of this surgical technique, and has learned this and other procedures from her mother firsthand. Over the years, Dr. Stepanian has performed this surgery both in Russia and in the U.S. She has presented at multiple international congresses and universities, and was elected to perform this surgery live at AAGL‘s International Congress. She is a co-author of multiple publications on the subject, including new classification of Müllerian Anomalies based on experiences with over 1,400 patients.

At Academia of Women’s Health & Endoscopic Surgery, we are highly specialized in müllerian anomalies, and we welcome women of all ages to come to us for a consultation to establish a plan for thorough, complex, and complete evaluation. We can recommend and perform a variety of successful treatment options. We can also provide much-needed comfort, understanding, and emotional guidance for women (often teens) and their families.

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About Dr. Stepanian

Dr. Assia Stepanian grew up in Moscow, the daughter of two illustrious physicians. In fact, much of her training was supervised by her mother, Dr. Leila Adamyan, the developer of some of the most advanced techniques and classifications used today in the practice of Minimally Invasive Gynecologic Surgery.